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This site is intended for US Population-Health Decision Makers or similar entities at hospitals and US oncology practices.

Quality Initiatives for Myelofibrosis (MF)

Myelofibrosis (MF) is a serious hematologic malignancy

MF is a Philadelphia chromosome-negative myeloproliferative neoplasm (MPN) marked by

  • bone marrow fibrosis
  • abnormal blood counts
  • extramedullary hematopoiesis
  • a significant symptom burden
  • shortened survival1

39% of patients with MF reach 5-year overall survival2,a

Most patients with MF have intermediate or high-risk disease, which is associated with shortened survival3,b

Clinical Consideration in Patients With MF

Myelofibrosis

is a chronic hematologic malignancy characterized by splenomegaly and symptom burden that can negatively impact quality of life4,5

Splenomegaly and symptom burden are present at diagnosis in many patients, and in one study, most patients were intermediate- or high-risk

within
1
YEAR
of diagnosis3,4,6,7

New or increasing

splenomegaly

is a marker of disease progression3,7

Splenomegaly can drive mortality as well as morbidity:

Larger spleen volume and constitutional symptoms related to splenomegaly have been associated with decreased overall survival7,8

Majority of Patients With MF Report Symptom Burden at Diagnosis4,6

Assessing patient symptoms at diagnosis is important
95% of patients reported 2+ MF-related symptoms at diagnosis based on a retrospective chart review of 180 patients with MF6*
The majority of patients with MF reported that symptoms impact quality of life4
Patients may not recognize that their symptoms are related to MF9
Changes in symptom status could be a sign of disease progression7

*Retrospective, observational study of symptom burden and splenomegaly in 180 patients with MF; 12 months or more of data were collected after diagnosis of MF in patients without splenomegaly (n=78) or after detection of splenomegaly in patients with splenomegaly (n=102). In patients with splenomegaly, splenomegaly was most often recorded at the time of diagnosis (median time from MF diagnosis to reported splenomegaly was 1 day).6,7

Recommended Monitoring

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Recommend Monitoring Patients with MF

The NCCN Guidelines® recommend:

  • Assessing symptom burden in patients with MF
  • Determining whether they are a transplant candidate
  • Monitoring response† to cytoreductive therapy
  • And monitoring for signs and symptoms† of disease progression10

† Every 3-6 months or more frequently as clinically indicated. This recommendation is for patients with lower-risk MF and for patients with higher-risk MF with platelets ≥50 x 109/L who are not transplant candidates.

For lower-risk MF, evaluation for allogeneic HCT is recommended for patients with low platelet counts or complex cytogenetics. For higher-risk MF, evaluation for allogeneic HCT is recommended for all patients.10

CONSIDER QualityInitiatives

Download a brochure on how to implement a QI in MF

Hb=hemoglobin; HCT=hematopoietic cell transplant; JAK=Janus kinase inhibitor; MF=myelofibrosis; MPN-SAF TSS=Myeloproliferative Neoplasm-Symptom Assessment Form Total Symptom Score; NCCN=National Comprehensive Cancer Network; PLT=platelet.

Footnotes
  1. a5-year overall survival rate was estimated using Surveillance, Epidemiology, and End Results (SEER) data obtained from population-based cancer registries of the US population and SEER*Stat Software version 8.3.2. The analysis included patients with initial/primary site diagnosis between years 2007-2011. Overall survival is defined as the proportion of patients surviving at the specified time interval after diagnosis.2
  2. bAs included in the Dynamic International Prognostic Scoring System (DIPSS) Plus tool. The DIPSS-Plus scoring system has been validated for risk stratification any time after a diagnosis of primary MF, but has been used clinically for risk stratification of patients with post-essential thrombocythemia MF and post-polycythemia vera MF. In the DIPSS-Plus scoring system, adverse points are assigned by first calculating the DIPSS score and then adding points for additional factors.

To calculate the DIPSS score, 1 point each is assigned to age >65 years, leukocyte count >25 × 109/L, circulating blast cells ≥1%, and constitutional symptoms (weight loss greater than 10% of the baseline value in the year preceding the primary MF diagnosis and/or unexplained persistent fever or excessive sweating), while 2 points are assigned for anemia (Hb <10 g/dL).

A DIPSS risk category is calculated, where 0 points = low risk, 1 or 2 points = intermediate-1 risk, 3 or 4 points = intermediate-2 risk, and 5 or 6 points = high risk. The DIPSS risk categories—low, intermediate-1, intermediate-2, and high risk—are given 0, 1, 2, or 3 points, respectively, in the DIPSS-Plus system, with an additional 1 point each for PLT count <100 × 109/L, red cell transfusion dependency, or unfavorable karyotype (complex karyotype or single or 2 abnormalities including +8, –7/7q-, i(17q), –5/5q-, 12p-, inv (3) or 11q23 rearrangement), resulting in a maximum possible score of 6.3

References: 1. Vannucchi AM, Guglielmelli P, Tefferi A. Advances in understanding and management of myeloproliferative neoplasms. CA Cancer J Clin. 2009;59(3):171-191. doi:10.3322/caac.20009 2. Surveillance, Epidemiology, and End Results (SEER) Program (www.seer.cancer.gov) SEER*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, November 2015 Submission (1973-2013 varying) - Linked to County Attributes - Total U.S., 1969-2014 Counties. Accessed January 4, 2017. 3. Gangat N, Caramazza D, Vaidya R, et al. DIPSS plus: a refined Dynamic International Prognostic Scoring System for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status. J Clin Oncol. 2011;29(4):392-397. doi:10.1200/JCO.2010.32.2446 4. Mesa R, Miller CB, Thyne M, et al. Myeloproliferative neoplasms (MPNs) have a significant impact on patients’ overall health and productivity: the MPN Landmark survey. BMC Cancer. 2016;16:167. doi:10.1186/s12885-016-2208-2 5. Cervantes F, Dupriez B, Pereira A, et al. New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and Treatment. Blood. 2009;113(13):2895-2901. 6. Mitra D, Kaye JA, Piecoro LT, et al. Symptom burden and splenomegaly in patients with myelofibrosis in the United States: a retrospective medical record review. Cancer Med. 2013;2(6):889-898. doi:10.1002/cam4.136 7. Emanuel RM, Dueck AC, Geyer HL, et al. Myeloproliferative neoplasm (MPN) symptom assessment form total symptom score: prospective international assessment of an abbreviated symptom burden scoring system among patients with MPNs. J Clin Oncol. 2012;30(33):4098-4103. doi:10.1200/JCO.2012.42.3863 8. Vannucchi AM, Kantarjian HM, Kiladjian JJ, et al. A pooled analysis of overall survival in COMFORT-I and COMFORT-II, 2 randomized phase III trials of ruxolitinib for the treatment of myelofibrosis. Haematologica. 2015;100(9):1139-1145. 9. Mesa RA, Miller CB, Thyne M, et al. Differences in treatment goals and perception of symptom burden between patients with myeloproliferative neoplasms (MPNs) and hematologists/oncologists in the United States: findings from the MPN Landmark survey. Cancer. 2017;123(3):449-458. doi:10.1002/cncr.30325 10. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Myeloproliferative Neoplasms V.1.2023. © National Comprehensive Cancer Network, Inc. 2023. All rights reserved. Accessed June 6, 2023. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.